A case of adult-onset Satoyoshi syndrome with gastric ulceration and eosinophilic enteritis.

BACKGROUND: The patient was misdiagnosed as having Sjögren's syndrome (on the basis of a lower-limb rash and dry eyes and mouth) in 1999, and then as having systemic lupus erythematosus (on the basis of hair loss and a high antinuclear antibody titer) in 2005. Total alopecia, muscular spasms and diarrhea developed over the following 2 years, and the patient experienced gastric ulceration in 2006. A rheumatologic opinion was sought in 2007. INVESTIGATIONS: Physical examination, CBC, glucose tolerance test, iron studies, HLA typing, immunological investigations and complete gastrointestinal investigations, including gastroscopy, colonoscopy and small bowel biopsy. DIAGNOSIS: Satoyoshi syndrome with autoimmune features (high levels of antinuclear antibody and antibodies to thyroid tissue) and malabsorption due to eosinophilic enteritis. This patient is only the fifth adult in the world reported to have Satoyoshi syndrome, and the first-reported adult case from South Africa. MANAGEMENT: The patient had only a transitory response to glucocorticoid treatment. Complete amelioration of symptoms resulted on two occasions when treated with intravenous immunoglobulin; however, the remissions only lasted for 6-8 weeks. More-intensive immunosuppression with azathioprine is currently being attempted.